Progressive supra-aortic stenosis in a young adult with the findings of Singleton Merten Syndrome.
BMJ Case Rep
; 20142014 Sep 05.
Article
em En
| MEDLINE
| ID: mdl-25193816
ABSTRACT
Singleton Merten Syndrome is an autosomal dominant disorder of unknown origin. Patients often present with muscular weakness, failure to thrive, abnormal dentition, glaucoma, psoriatic skin lesions, aortic calcification and musculoskeletal abnormalities. In this case, we present a young girl with a history of aortic root replacement, who had an unusual progressive supra-aortic stenosis managed with urgent surgery during the course of the syndrome. Cardiovascular involvement needs special attention, since it is the major cause of mortality along with rhythm disturbances in the course of Singleton Merten Syndrome.
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1
Base de dados:
MEDLINE
Assunto principal:
Osteoporose
/
Doenças da Aorta
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Estenose da Valva Aórtica
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Odontodisplasia
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Hipoplasia do Esmalte Dentário
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Calcificação Vascular
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Metacarpo
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Doenças Musculares
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article